NK cell compartment in the peripheral blood and spleen in adult patients with primary immune thrombocytopenia.

Immune thrombocytopenic purpura (ITP) is a disease characterized by antibody-mediated platelet destruction. The T- and B-cell subsets have been extensively studied in primary ITP, but the NK cell compartment has been less thoroughly explored. We investigated the NK cell receptor repertoire and the functionality of NK cells in the peripheral blood and spleen in patients with primary ITP. An immunophenotypic analysis of peripheral blood lymphocytes from patients revealed that the numbers of CD19+ B lymphocytes, CD4+ and CD8+ T lymphocytes and CD3-CD56+ NK cells were within the normal range. No major alteration to the expression of distinct inhibitory or activating NK cell receptors was observed. The functionality of NK cells, as evaluated by their ability to degranulate in conditions of natural cytotoxicity or antibody-dependent cell cytotoxicity (ADCC), was preserved in these patients. By contrast, these stimuli induced lower levels of IFNγ production by the NK cells of ITP patients than by those of healthy controls. We then compared the splenic NK cell functions of ITP patients with those of cadaveric heart-beating donors (CHBD) as controls. The splenic NK cells of ITP patients tended to be less efficient in natural cytotoxicity conditions and more efficient in ADCC conditions than control splenic NK cells. Finally, we found that infusions of intravenous immunoglobulin led to the inhibition of NK cell activation through the modulation of the interface between target cells and NK cells.

Description of Walkeriella miraculosa, gen. n. et sp. n. from South-East Peru (Lepidoptera: Hepialoidea: Hepialidae).

Walkeriella miraculosa gen. n. et sp. n. (Lepidoptera: Hepialoidea: Hepialidae) is based on two specimens collected from rainforest southwest of Puerto Maldonado, Tambopata, Madre de Dios department, southeastern Peru. The distinct genitalia and wing venation separate this taxon from other genera of Hepialidae although four derived characters states are shared in common with the cibyrine clade. The holotype male is deposited in the collection of the Museo de Historia Natural, Lima, Peru.

[Long-lasting thrombocytopenia induced by glycoprotein IIb/IIIa inhibitor]. / Thrombopénie sévère et prolongée induite par un inhibiteur de la glycoprotéine IIb/IIIa.

INTRODUCTION: Glycoprotein IIb/IIIa inhibitors (anti-GPIIbIIIa) prevent platelet binding to fibrinogen. Transient sometimes-severe thrombocytopenia is a well-known side effect. OBSERVATION: A 71-year-old patient presented severe thrombocytopenia after the administration of tirofiban (anti-GPIIbIIIa). Corticosteroid treatment was initiated at day 10 because of persistence of severe thrombocytopenia with poor platelet transfusion efficacy. Corticosteroid treatment led to platelet recovery evoking an immune mediated mechanism for thrombocytopenia. CONCLUSION: Anti-GPIIbIIIa are associated with a risk of dramatic thrombocytopenia. The underlying mechanism is poorly understood. The management of these usually transient thrombocytopenias is based on platelet transfusion. As report here, in some cases persistent thrombocytopenia can respond to corticosteroids.

[IgG4-related disease treatment in 2014: Update and literature review]. / Traitement de la maladie associée aux IgG4 en 2014 : mise au point et revue de la littérature.

IgG4-related disease is an inflammatory disorder characterized by a polyclonal lymphoplasmacytic tissue infiltrate, with numerous IgG4+ plasmocytes, evolving toward fibrosis. The disease is heterogeneous and affects several tissues and organs synchroneously or metachroneously. Both the fibrosis and the tumor forming characteristics of the disease can be responsible of irreversible tissue damage. For these reasons treatment is usually necessary. A dramatic response is usually observed with steroid treatment but relapses are frequent. Immunosuppressive agents and rituximab are used as second line treatments. We review here previous studies on treatment and suggest general recommendations for the treatment and follow up of patients with IgG4-related disease.

[IgG4-related systemic disease: emergence of a new systemic disease? Literature review]. / Maladie systémique associée aux IgG4 : une pathologie émergente ? Mise au point et revue de la littérature.

Hyper-IgG4 syndrome, or IgG4-related systemic disease (IgG4-RSD), has been recently characterized by the association of a focal or diffuse enlargement in one or more organs, elevated levels of serum IgG4 and histopathological findings including "storiform" fibrosis and prominent infiltration of lymphocytes and IgG4-positive plasma cells. Pancreas was the first organ involved with sclerosing pancreatitis (or autoimmune pancreatitis). Since this first description, many extrapancreatic lesions have been described, even in the absence of pancreatitis and include sialadenitis, lacrimal gland inflammation, lymphadenopathy, aortitis, sclerosing cholangitis, tubulointerstitial nephritis, retroperitoneal fibrosis or inflammatory pseudotumors. Multiorgan lesions can occur synchronously or metachronously in a same patient, usually after 50 years of age. They all share common histopathological findings. The disease often responds well to corticosteroid therapy. In this literature review on IgG4-RSD, we present historical, epidemiological and clinical characteristics, and we review the biological and histological diagnostic criteria. To date there is no international validated diagnostic criteria. Pathophysiological hypothesis and therapeutic approaches are also discussed.

[Carotid-cavernous sinus fistula: an unusual cause of exophthalmia. Report of three cases]. / Une cause inhabituelle d'exophtalmie uni- ou bilatérale : les fistules artérioveineuses carotidocaverneuses. Trois observations.

INTRODUCTION: Exophthalmia can be associated with several disorders. Although unusual, a carotid-cavernous fistula should be ruled out systematically as it may be associated with severe complications. CASE REPORTS: We report three cases associated with both types of carotid-cavernous fistula that highlight the clinical presentation and diagnostic process. CONCLUSION: A carotid-cavernous fistula should be systematically ruled out in patients with a uni- or bilateral exophthalmia. Careful examination with auscultation of the ocular globe contributes greatly to the diagnosis that may be confirmed by MRI or arteriography.

[Acute bowel intussusception revealing celiac disease: a new case and literature review]. / Invagination intestinale aiguë révélant une maladie cœliaque : à propos d'un cas et revue de la littérature.

INTRODUCTION: Acute bowel intussusception is a rare manifestation in adult, which mainly involves the small intestine. Celiac disease is a frequent small bowel disease that is largely undiagnosed in adults. We report a patient in whom spontaneously regressive small bowel intussusception was the presenting manifestation of celiac disease. CASE REPORT: A 40-year-old man was admitted for a right-sided iliac abdominal pain related to a small bowel intussusception. Laparotomy ruled out a digestive tumor. Persistence of diffuse abdominal pain associated with progressive and unexplained weight loss for several months led to the diagnosis of celiac disease, which was confirmed by the presence of specific serum autoantibodies and histological duodenal villous atrophy. CONCLUSION: The association between small bowel intussusception and celiac disease does not seem to be fortuitous. Based on this report and the literature review, we suggest that celiac disease can favour small bowel intussusception, even in adulthood. Therefore, diagnosis of celiac disease must be discussed in the presence of unexplained intussusception.